The modified natural history of congenital heart disease

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1 The modified natural history of congenital heart disease Matthias Greutmann, MD Adult Congenital Heart Disease Program University Hospital Zurich, Switzerland

2 Are we ready for the future? Matthias Greutmann, MD Adult Congenital Heart Disease Program University Hospital Zurich, Switzerland

3 Objectives Natural history of complex CHD Effect of surgical modificantion Current situation Outlook and needs for the future

4 Survival Chance of survival to adulthood with complex CHD % Modified from Dr. H. Kaemmerer, Munich

5 Norwood

6 Transposition of the great arteries

7 Survival (%) Natural history of TGA PFO only ASD VSD + PS Age (years) Liebman, Circ 1969

8 Survival (%) Modified history of TGA Atrial switch Arterial switch - early Arterial switch contemporary Age (years) Liebman, Circ 1969 Williams, EJCTS 2003 Wong, HLC 2008

9 Improved Survival % BT-Shunt Fallot-repair 70 Atrial switch Norwood Arterial switch Fontan-operation Modified from Dr. H. Kaemmerer, Munich

10 Number of patients Number of patients with CHD children adults Marelli, Circ 2007

11 Number of patients Shift to Adults: complex CHD children adults Marelli, Circ 2007

12 Prevalence All congenital heart defects: 4.1 / 1000 adults Complex defects: 0.38 / 1000 adults Marelli, Circ 2007

13 Number of adults with CHD Switzerland 7.8 Mio Europe 730 Mio

14 Mortality Shift Age at death (years) <1 30% 20% 10% 0% 0% 10% 20% 30% Proportion of all deaths CHD deaths General population Khairy, JACC 2010

15 Mortality Shift Age at death (years) <1 30% 20% 10% 0% 0% 10% 20% 30% Proportion of all deaths CHD deaths General population Khairy, JACC 2010

16 Who are these patients?

17 Case 1: Female, 23 years old Left atrial isomerism single right ventricle, common atrium, severe pulmonary stenosis Palliated with left modified BTshunt

18 Palliated cyanotic CHD RV Hypoplastic LV Single atrium

19 Case 2: Male, 45 years old Tricuspid atresia Classic Glenn shunt Fontan operation Now: Failing Fontan

20 Fontan procedure tricuspid atresia single LV giant RA LA

21 Case 3: male, 23 year old Transposition of the great arteries RV LV PV-baffle Atrial switch

22 end-stage heart failure

23 What is the outcome of adults with complex CHD?

24 506 deaths among patients 18% 13% 3% 34% 32% SCD Cardiac - not sudden Perioperative Noncardiac Unknow n AHA abstract, 2010

25 deaths Deaths per decade

26 deaths Total patients Deaths vs. total patients

27 Disease Complexity No of deaths simple moderate severe

28 Mode of death Simple Moderate Severe % 100 Non- Cardiac Cardiac p < 0.001

29 Age of death Changes in age of death p = 0.7 p = 0.01 p = Simple Moderate Severe

30 Most common entities Fontan palliation (9%) Tetralogy of Fallot (11%) Subaortic right ventricles (13%) Cyanotic defects / Eisenmenger (29%) 13% of all patients but 62% of all deaths

31 Number of deaths Most common entities Cyanotic Fontan TOF TGA Cyanotic Fontan TOF TGA Cyanotic Fontan TOF TGA

32 Number of pts Active & alive Patients Tetralogy of Fallot TGA Cyanotics Fontan

33 Alive vs. deceased patients % 80% 60% 40% alive death 20% 0% Tetralogy Fontan TGA Cyanotics

34 Conclusions I There is an emerging population of young adult survivors with complex CHD These patients are at high risk of premature death at young age

35 Outlook and needs Transplantation Communication

36 Is transplantation the solution?

37 Number of adults with CHD Switzerland 7.8 Mio Europe 730 Mio

38 506 deaths among patients 18% 13% 3% 34% 32% SCD Cardiac - not sudden Perioperative Noncardiac Unknow n AHA abstract, 2010

39 deaths Total patients No. of Transplantations

40 No. of Transplantations Year of Transplant Listings Transplants Davies, Circ 2011

41 Reasons not to transplant CHD

42 % survival Outcome after Transplantation Non-CHD CHD Fontan Years Davies, Circ 2011 Lamour, JACC 2009

43 Communication Do we communicate enough?

44 Percent What do patients think Students CHD mod. CHD - complex < >95 Self-estimated life expectancy Reid, JACC 2006

45 Communication 200 Adults with CHD 35 ± 15 years, 80% mod. or great disease complexity 48 Health care provider Tobler et al, IJC 2010

46 Patients Have you ever discussed EOL planning with a member of your medical team? % % No 1% Yes

47 Patients Do you think a member of your medical team should bring up EOL discussions? 80 77% % % 0 No Yes

48 Provider How often do you typically discuss EOL issues with outpatients? 89% % % 49% 0 Simple M oderate Great p < compared to patient group

49 Patients Do you think a member of your medical team should bring up EOL discussions? % 79% 77% % Simple M oderate Great

50 Timing When do you think is the best time to bring up EOL discussions with patients? Patients Provider % w hile healthy first complication admitted / dying w hile healthy Before getting sick, while healthy first complication first life-threatening illness or complication admitted / dying first admitted with life-threatening complication or dying p < 0.001

51 Are we ready for the future? Novel therapeutic approaches Better prognostication Better communication

52 Thank you! Matthias Greutmann, MD Adult Congenital Heart Disease Program University Hospital Zurich, Switzerland

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